The term "sarcoidosis" refers to a systemic disease. Various organs can be involved in the pathological process, but the respiratory system is most often affected. Against the background of the course of the disease in healthy tissues, the formation of specific granulomas occurs. The exact causes of pulmonary sarcoidosis are currently unknown. However, one of the triggering factors is age. Most often, the pathology is diagnosed in people from 20 to 35 years old. With lung sarcoidosis, the prognosis for life directly depends on the timeliness of going to the doctor. There are several methods of treating an ailment, and if they are ineffective, the question of the advisability of surgical intervention is being addressed.
Pathogenesis
It is important to know that lung sarcoidosis is a disease characterized by a multi-organ course. Under the influence of any provoking factors, damage to the alveolar tissue occurs. This pathological process is accompanied by the onset of pneumonitis of an interstitial nature. Alveolitis often develops, followed by the formation of sarcoid granulomas. They are formed in interlobar sulci, as well as in peribronchial and subpleural tissues.
Over time, the formed granulomas dissolve independently or degenerate into a hyaline cell-free mass. Against the background of the progression of pathology, the ventilation function is disturbed. Lymph nodes begin to exert pressure on the walls of the bronchi, due to which violations of an obstructive nature often develop. Less often, the formation of atelectasis and hypoventilation sites occurs.
As the granulomas grow, they merge with each other, forming large foci of pathology. Against the background of this process, lung function is impaired. It is at this stage that pronounced clinical manifestations of the disease appear. With lung sarcoidosis, the prognosis for life depends on the nature of the course of the pathology. As mentioned above, granulomas can resolve on their own or, conversely, cause fibrotic changes in the affected organ.
The International Classification of Diseases (ICD-10) assigns code D86.0 to sarcoidosis of the lungs.
Etiology
Currently, the exact cause of the development of the disease is unknown. Scientists have been conducting research for more than a century, but they still have not yielded results. In the process of testing, several theories of the onset of the disease appeared. In each of them there is a certain mechanism for the development of pathology.
Theories of lung sarcoidosis:
- Infectious. The causative agent of the disease is one or another pathogenic agent. Penetrating into the body, they provoke the formation of an immune response. The protective system produces antibodies against the pathogen that freely enter the bloodstream and circulate with it. If they move within the body for a long time, it causes certain disturbances at the cellular level. In the presence of a hereditary predisposition in humans, as a rule, a systemic pathology develops. Scientists believe that people at risk are those who have experienced the following diseases in the past: tuberculosis, chlamydia, tick-borne borreliosis, gastric ulcer, rubella, hepatitis C, adenovirus infection, herpes.
- Theory of contact transmission. Its essence is that most people with sarcoidosis previously interacted with patients. Often, in practice, there are cases of family contact, while relatives have a pathology at different times. This theory arose after research on mice. In addition, there are several cases of infection after organ transplantation.
- Theory of the influence of external factors. At risk are people whose professional activities involve frequent contact with harmful chemical compounds. In addition, dust is a likely cause of the disease. According to studies, in people who regularly inhale it (miners, rescuers, etc.), sarcoidosis of the lungs is diagnosed 4 times more often. The most harmful are particles of aluminum, beryllium, copper, gold, zirconium, cobalt and titanium.
- Theory of heredity. It is possible that people have defective genes in their bodies. They, in turn, encode abnormal proteins. But at the same time, the process of the development of the disease itself starts only under the influence of some provoking factor, for example, after contact with the patient.
- Medication. In practice, there have been cases when the formation of granulomas occurred against the background of prolonged use of certain drugs, in particular, antiretroviral drugs and agents based on interferon. It is rather difficult to confirm this theory, since these medications need to be taken for a long time with diseases that themselves can cause sarcoidosis. A prime example is AIDS.
Thus, the exact cause of the development of the disease is unknown. Currently, the theory of heredity is considered the main one, and all other factors are considered as provoking.
Clinical manifestations
It is important to know that lung sarcoidosis is a disease that is systemic. It has several forms, but each of them has common features.
At the initial stage of the development of the disease, the following symptoms appear:
- General weakness. Up to 80% of patients complain of malaise. Weakness appears against the background of metabolic and biochemical processes in the body. Most often, she bothers in the morning. Patients note that after waking up they do not feel a surge of strength, even after a long night's sleep, they do not recover.
- Fever. This symptom appears quite rarely. It occurs against the background of the course of an acute inflammatory process.
- Weight loss. It does not occur abruptly, but gradually. Weight loss due to metabolic disorders and the presence of chronic processes that are difficult to treat.
- Dyspnea. This symptom appears with sarcoidosis of the lungs and lymph nodes in them. The severity of shortness of breath increases after physical exertion, which is due to the increased need for tissue oxygen. To ensure it, the bronchi expand, but due to granulomas and enlarged lymph nodes, a sufficient amount of air does not enter the lungs anyway.
- Cough. It occurs with irritation of nerve fibers and compression of the respiratory passages. At the initial stages of the development of the disease, the cough is dry. Over time, it begins to be accompanied by severe pain. This is due to the fact that in the later stages, pleura is involved in the inflammatory process. In addition, if granulomas formed near her, breathing also becomes painful.
- Chest discomfort. Minor painful sensations are detected in a third of patients. However, they do not radiate to other parts of the body.
If there is a violation of the function of gas exchange, ventilation or pulmonary blood flow, signs of respiratory failure appear. It is important to remember that lung sarcoidosis is a disease that can affect any organ. With untimely treatment, all kinds of complications develop.
Forms and severity
According to localization and radiological indicators, distinguish:
- Damage to the intrathoracic lymph nodes.
- Sarcoidosis of the lungs.
- Damage to the respiratory system.
- Sarcoidosis of the lungs and intrathoracic lymph nodes.
- Generalized form of the disease.
- Sarcoidosis of the lymph nodes.
Since the disease is systemic, granulomas can form in various tissues. However, in practice, both the lungs and lymph nodes are more often involved in the pathological process. The generalized form is considered the most severe, since it affects almost all systems.
The ICD-10 code for lung sarcoidosis with lymph node involvement is D86.2. In the classification, several more forms of the disease are indicated. The ICD-10 code for lung sarcoidosis of other combined locations is D86.8. In some cases, the doctor cannot, according to available signs, attribute the pathology to one form or another. In this situation, it is necessary to indicate another ICD code - “Unspecified lung sarcoidosis” (D86.9).
It is also customary to divide the course of the disease into stages. The degree of lung sarcoidosis:
- First one. At this stage, granulomas are actively formed, forming lesions. But the diagnosis at this stage is still difficult to make. At this time, the first signs of the disease begin to appear.
- The second one. This is the stage of remission. It is characterized by a slowdown in the growth of old granulomas. New ones are practically not formed. At this stage, it is very easy to diagnose with a biopsy. Symptoms of the disease do not disappear, but the patient’s condition does not significantly worsen. The appearance of new signs for this stage is not characteristic.
- The third. This is a stable course of pathology. Granulomas gradually increase in size. In the foci of pathology, you can find areas of necrosis, which becomes the cause of the deterioration of the patient's well-being. The disease develops slowly, but lung sarcoidosis is not completely cured.
For independent resorption of granulomas, a reverse development is characteristic.
Diagnostics
It is quite difficult to immediately detect the disease, since its symptoms are similar to the clinical manifestations of more common ailments. In order for the doctor to be able to make the correct diagnosis, the patient must undergo a comprehensive examination, including:
- General blood analysis. The following indicators are clinically significant: red blood cells, white blood cells, eosinophils, lymphocytes, monocytes, ESR.
- A blood test for ACE. This is an enzyme that can be produced in large quantities by the cells that make up granulomas.
- Blood test for calcium.
- Examination of liquid connective tissue for tumor necrosis factor alpha.
- Sample Kveim - Silzbach. Its essence is the introduction to the patient of lymphatic tissue affected by the disease. Then the doctor evaluates the result according to the condition of the skin.
- Mantoux test.
- Blood test for copper.
- Roentgenography.
- CT
- MRI
- Scintigraphy (radionuclide study).
- Ultrasound
- Spirometry.
- ECG.
- Electromyography.
- FGDS.
- Fundus examination.
Thus, it is worthwhile to understand that pulmonary sarcoidosis is a pathology that is difficult to detect, it takes a long time. Nevertheless, the doctor can draw up a treatment regimen only on the basis of the results of a comprehensive examination.
Conservative Therapies
According to reviews, lung sarcoidosis is not a sentence. If you strictly follow all the doctor's recommendations, it is possible to achieve the onset of stable remission and forget about the disease.
There are several regimens for treating pulmonary sarcoidosis. Reviews of doctors and their patients confirm that monotherapy is the most effective at an early stage of the development of the disease. It involves the introduction of only one drug. As a rule, doctors prescribe Methotrexate, Pentoxifylline, or Alpha Tocopherol.
Another treatment regimen is combined. In its framework, the simultaneous administration of "Chloroquine" and glucocorticosteroids or "Alpha-tocopherol" and "Pentoxifylline" is shown. If necessary, the doctor prescribes taking drugs related to the NSAID group. With damage to other organs, symptomatic therapy is indicated.
Sarcoidosis of the lungs and intrathoracic lymph nodes is considered more severe. In this case, the disease can be resistant to hormonal drugs. In this situation, irradiation of the affected area (chest) is indicated. As a rule, the disease recedes after 3-5 procedures.
Doctors often recommend a diet for patients. There is no therapeutic nutrition for sarcoidosis, but, according to statistics, regular fasting in 75% of cases stops the development of the pathological process. This method should be practiced only in a hospital and under the supervision of doctors.
The goal of treating sarcoidosis is also to prevent the development of complications. Doctors recommend ventilating the room more often, doing wet cleaning regularly, and avoiding stressful situations and hypothermia.
Surgical intervention
Surgical treatment is carried out only in isolated cases. This is due to the fact that the removal of the affected lymph nodes is a rather large-scale intervention, and granulomas, even in their absence, can subsequently form again.
An operation is performed only if there is no other way to save the patient's life. Also, the need for surgical intervention is required when developing dangerous complications of the disease.
Effects
Pulmonary sarcoidosis alone, in extremely rare cases, is the cause of the death of the patient. But the disease can trigger the development of life-threatening complications.
The most common effects of sarcoidosis are:
- Collapse of the lung. It occurs if the integrity of the pleura is violated against the background of growth of granulomas. As a result, its pressure is equalized with atmospheric pressure. A logical consequence is the compression of the lung. If you do not provide first aid to a person in time, he will die in a fairly short time.
- Bleeding. Develop against vascular damage. As a rule, the outflow of liquid connective tissue stops independently. An exception is when the liver is affected by sarcoidosis.
- Frequent episodes of pneumonia. This complication most often occurs against the background of hormonal therapy. Glucocorticosteroids contribute to the weakening of the body's defenses, making it susceptible to infectious agents.
- The formation of calculi in the kidneys. Stones form due to the large amount of calcium in the blood. In the pelvis, it binds to other trace elements and forms salts.
- Heart rhythm disturbance. One of the most dangerous complications. Against the background of heart rhythm disturbance, the brain begins to suffer from a lack of oxygen. As a result, nerve cell death occurs.
- Fibrosis. The lung cells are modified, so that the organ ceases to function normally. In this case, the only treatment is transplantation.
In addition, almost all patients with sarcoidosis have problems of a psychological nature. They arise amid a deterioration in the quality of life.
Forecast
Sarcoidosis of the lungs is a rather dangerous disease, but with timely medical attention it is possible to achieve the onset of stable remission. According to statistics, about 3% of patients die. In 70% of cases, it is possible to achieve prolonged remission.
It is important to remember that the outcome of a disease depends on many factors. It is impossible to completely get rid of the disease, but it is possible to avoid exacerbations with strict adherence to the doctor’s recommendations.
Finally
Lung sarcoidosis is a pathology characterized by the formation of specific granulomas in healthy tissue. Currently, the exact causes of the development of the disease are unknown, but the theory of hereditary predisposition is the main one. With timely access to a doctor, the prognosis is usually favorable. However, it is important to be prepared for the fact that the diagnostic process will take a long time. This is due to the fact that the symptoms of the disease are similar to the signs of more common pathologies.
The International Classification of Diseases (ICD-10) assigns code D86.0 to sarcoidosis of the lungs.